Kimmelstiel-Wilson Syndrome Group O

KIMMELSTIEL – WILSON SYNDROMEGENERAL OBJECTIVE

• To comprehensively understand Kimmelstiel-Wilson Syndrome.Specific Objectives

1. To correlate the patient’s overall history with the disease process.2. To identify pertinent physical examination results evident in the patient.3. To analyze the laboratory and diagnostic studies which will justify the diagnosis.4. To review the anatomy and physiology of the systems involved.5. To thoroughly explain the pathophysiology of the disorder including risk factors and

clinical manifestations.6. To evaluate the nursing and medical management rendered to the patient through the

Nursing Care Plan.7. To discuss the prognosis of the patient and the recommendations to combat or

decelerate the progression of the disease. INTRODUCTION

History Diabetic nephropathy (nephropatia diabetica), also known as Kimmelstiel- Wilson

syndrome and intercapillary glomerulonephritis, is a progressive kidney disease caused by angiopathy of capillaries in the kidney glomeruli. It is characterized by nephrotic syndrome and nodular glomerulosclerosis. It is due to longstanding diabetes mellitus, and is a prime cause for dialysis in many Western countries. Discovered by British physician Clifford Wilson (1906-1997) and Germanborn American physician Paul Kimmelstiel (1900-1970) and was published for the first time in 1936.

CausesThe exact cause of diabetic nephropathy is unknown, but it is believed that

uncontrolled high blood sugar leads to the development of kidney damage. In some cases, your genes or family history may also play a role. Not all persons with diabetes develop this condition. Each kidney is made of hundreds of thousands of filtering units called nephrons. Each nephron has a cluster of tiny blood vessels called a glomerulus. Together these structures help remove waste from the body. Too much blood sugar can damage these structures, causing them to thicken and become scarred. Slowly, over time, more and more blood vessels are destroyed. The kidney structures begin to leak and protein (albumin) begins to pass into the urine.

Persons with diabetes who have the following risk factors are more likely to develop this condition: African American, Hispanic, American Indian or Asian origin Family history of kidney disease or high blood pressure, poor control of blood pressure, poor control of blood sugars, Type 1 diabetes before age 20, smoking. Diabetic nephropathy generally goes along with other diabetes complications including high blood pressure, retinopathy, and blood vessel changes.

Prevalence and Incidence RateDiabetes has become the primary cause of end-stage renal disease (ESRD), and the

incidence of type 2 diabetes mellitus continues to grow worldwide. Approximately 44% of new patients entering dialysis are diabetics. Early diagnosis of diabetes and early intervention are critical in preventing the normal progression to renal failure seen in many type 1 and a significant percentage of type 2 diabetics. The prevalence of diabetes is higher in certain racial and ethnic groups, affecting approximately 13% of African Americans, 9.5% of Hispanics, 15% of Native Americans and 15% of Asians, primarily with type 2 diabetes. Approximately 20% to

30% of all diabetics will develop evidence of nephropathy, although a higher percentage of type 1 patients progress to ESRD.

EpidemiologyThe syndrome can be seen in patients with chronic diabetes (15 years or more after

onset), so patients are usually of older age (between 50 and 70 years old). The disease is progressive and may cause death two or three years after the initial lesions, and is more frequent in men. Diabetic nephropathy is the most common cause of chronic kidney failure and end-stage kidney disease. People with both type 1 and type 2 diabetes are at risk. The risk is higher if blood-glucose levels are poorly controlled. Further, once nephropathy develops, the greatest rate of progression is seen in patients with poor control of their blood pressure. Also people with high cholesterol level in their blood have much more risk than others.

Signs & SymptomsEarly signs and symptoms of kidney disease in patients with diabetes are typically

unusual. However, a vast array of signs and symptoms listed may manifest when kidney disease has progressed: Swelling, usually around the eyes in the mornings; later, general body swelling may result, such as swelling of the legs; Foamy appearance or excessive frothing of the urine; Unintentional weight gain (from fluid accumulation); Fatigue; Frequent hiccups; General ill feeling; Generalized itching; Headache; Nausea and vomiting; Poor appetite; Weakness, paleness, and anemia; Ankle and leg swelling, leg cramps; Going to the bathroom more often at night High blood pressure

Tests and diagnosisLaboratory tests that may be done include: BUN (Blood Urea Nitrogen) Serum

creatinine The levels of these tests will increase as kidney damage gets worse. Other laboratory tests that may be done include: 24-hour urine protein Blood levels of phosphorus, calcium, bicarbonate, and potassium Hemoglobin Hematocrit Protein electrophoresis - urine Red blood cell (RBC) count.

ComplicationsPossible complications include: hypoglycemia (from decreased excretion of insulin)

rapidly progressing chronic kidney failure, end-stage kidney disease, hyperkalemia, severe hypertension, complications of hemodialysis, complications of kidney transplant, coexistence of other diabetes complications, peritonitis (if peritoneal dialysis used), increased infections.

About 20% of clients with type II are found to have diabetes nephropathy 5-10 yrs after diagnosis. A consequence of microangiopathy, nephropathy involves damage to and eventual obliteration of the capillaries that supply the glomeruli of the kidney. This damage leads in turn to a complex a pathologic changes and manifestations which includes intercapillary glomerulosclerosis, nephrosis, gross albuminuria and hypertension. Risk factors include poor glycemic control, duration of disease and hypertension. With worsening of the nephrosis, chronic renal failure ensues. Unless the client can be maintained with hemodialysis or receives a renal transplant, uremia eventually causes death. Diabetic nephropathy cannot be cured.

However prompt and adequate intervention for renal and bladder infection can prevent these causes of renal failure. Unsuccessfully treated nephropathy progresses to end stage renal disease. Treatment at this point includes hemodialysis, peritoneal dialysis or kidney transplantation.

Treatments and drugsThe goals of treatment are to keep the kidney disease from getting worse and prevent

complications. This involves keeping the blood pressure under control (under 130/80).

Kimmelstiel-Wilson Syndrome Group O

Controlling high blood pressure is the most effective way of slowing kidney damage from diabetic nephropathy. The doctor may prescribe the following medicines to lower the blood pressure: Angiotensin-converting enzyme (ACE) inhibitors and Angiotensin receptor blockers (ARBs). These drugs help reduce the amount of protein in the urine. Many studies have suggested that a combination of these two types of drugs may be best.

It is also very important to control lipid levels, maintain a healthy weight, and engage in regular physical activity. Closely monitor the blood sugar levels. Doing so may help slow down kidney damage, especially in the very early stages of the disease. Change diet to help control blood sugar. The doctor may also prescribe medications to help control the blood sugar. Dosage of medicine may need to be adjusted from time to time. As kidney failure gets worse, our body removes less insulin, so smaller doses may be needed to control glucose levels.

Urinary tract and other infections are common and can be treated with appropriate antibiotics. Dialysis may be necessary once end-stage renal disease develops. At this stage, a kidney transplant must be considered. Another option for patients with type 1 diabetes is a combined kidney-pancreas transplant.

COMPREHENSIVE HEALTH HISTORY

Patient’s Profile

NAME: Patient LoveAGE: 60SEX: FemaleBIRTHDAY: July 23, 1949CIVIL STATUS: WidowedBIRTHPLACE: Balangiga, Eastern SamarPERMANENT ADDRESS: Brgy. 59, Picas, Sagkahan, Tacloban CityNATIONALITY: FilipinoRELIGION: Roman CatholicOCCUPATION: HousewifeDATE OF ADMISSION: January 7, 2010ATTENDING PHYSICIAN: Dr. Aloha SaballeCHIEF COMPLAINT: Bipedal edema, pallor, easy fatigabilityDIAGNOSIS: Diabetic Nephropathy with anemia

History of Present Illness

On January 2, 2010, patient noticed that her feet were swelling (about 4mm). No other associated symptoms were noted. She took Diamicron as her maintenance medication for Diabetes. No medical consultation was done.

On January 7, 2010, patient was scheduled for a regular check-up with Dr. Aloha Saballe and when checked, the result of her blood glucose level was 220 mg/dl and her blood pressure reading was 220/140mmHg. Dr. Saballe advised the patient to stay and be confined at the EVRMC for further evaluation and management.

Medical History

Patient was diagnosed to be hypertensive when she was 40 years old. She was prescribed with Olmezar 20mg OD as a maintenance drug.

On December 2002, as recalled by the patient, she was confined at the EVRMC due to excessive urination and body weakness. She was diagnosed as having Diabetes Mellitus Type 2. Diamicron was given as her medication for her everyday maintenance to which she was compliant. She was advised measures to control her blood glucose level and a scheduled monthly check-up.

Childhood Illnesses: Measles, mumps, chickenpox.

Allergies: Patient had no known allergies.

Alcohol: Patient drinks 1-2 medium-sized glasses of tuba once every month.

Smoking: Patient is a non-smoker.

Family History

Patient love is the 8th child among 10 siblings. To date, only 3 of them are still alive. Her father was hypertensive and died with unknown cause. Her mother was diagnosed to have DM type II. Four of her siblings were known diabetics which were not properly managed. All of her siblings were hypertensive.

Heredofamilial Diseases:

Paternal Side: (+) Hypertension, DM

Maternal Side: (+) Hypertension, DM, Bronchial asthma

Personal/Psychosocial History

Patient Love was born and raised in Balangiga, Eastern Samar. She is an elementary graduate. She got married at age 22 to a PUJ driver from Tacloban and since their marriage, they have resided in the city already. They were blessed with four children, 2 boys and 2 girls all of whom have families of their own already. Her husband passed away in 2006 at the age of 56 due to a heart attack. Patient love is a housewife and has a small sari-sari store which helps augment the family’s income. At present, her youngest daughter and her family lives with Patient love.

Activities: Typical day: Arises at 5 AM, prepares breakfast, cleans the house, tends her store, watches over 2 grandchildren, prepares lunch, takes afternoon naps, gardens, prepares dinner, watches TV, sleeps at 8 PM.

Diet: Eats 3 times per day and usual diet consists of 2 cups of rice, fish, sometimes meat, vegetables and 8 glasses of water in one day. Before diagnosed to have diabetes, she consumes 500ml of Coke everyday and is fond of eating sweets such as kalamay on fried banana or camote. She puts a minimum of 2 tbsp of sugar in her coffee everyday. Her day was

Kimmelstiel-Wilson Syndrome Group O

not complete without something sweet in her meal. She modified her diet after she was diagnosed to have DM.

Hobbies:

Her sari-sari store keeps her busy and she takes care of 2 grandchildren who are living with her. These keep her busy. They sometimes spend weekends by the beach.

Interpersonal Relationship:

She has not encountered any problem in dealing with other people. She and her children, in-laws, and grandchildren have very good relationships and they share an open line of communication and has not encountered any major argument.

Health Perception:

She expressed that she has lived with her condition for a long time now. She tries her best to comply with her medication regimen and to follow what her doctor has prescribed her such as diet modification, exercise, and regular check-up. She is still saddened though because she does not live a normal life anymore. There are a lot of restrictions to her which she has to stick by religiously so as to extend her life. She still hopes that her condition gets better. She just prays hard and lifts everything to God.

GORDON’S HEALTH FUNCTIONING PATTERN

Health perception and management

Subjective: Patient verbalized, “Han bata pa ako, waray ako kaospital bisan makausa. Yana naman la ini nga tikalagas na ako. 40 years old ako han nasabtan nga high blood ngayan ak. Gintagan ak hin Olmezar 20 mg nga tutumarun kada adlaw pampahamubo hin BP. Han December 2002, gindara ak ha ospital kay duro na an ak pag-inihi ngan nanluluya ak. Yakan han ak doctor hataas daw kuno ak sugar ha dugo, may diabetes daw ako. Gintagan liwat ak hin Diamicron 80mg nga nakakaduha pagtumar ha usa ka adlaw. Diri na gihap ak ginpapakaon hin magtam-is ngan duro nga karne. Ngatanan nga ak ginkakaon, sinukol. Ginpapadamu pagkaon hin utan nga linaw-agan. Ginpapaglakat-lakat gihap ako para exercise. Maupay nala liwat nga adi tak mga apo kay nakakagi-os ako kay ako man it nag-aalaga ha ira. Gintatalinguha ko nga masunod ini ngatanan nga ginyakan han ak doctor para man liwat ini hit akon kaupayan ngan para mag-iha pa it ak kinabuhi. Nababaraka gad gihapon ako kay diri ako maaram kun ano it mahihinabo ha akon tungod hini nga ak sakit. Pero kakayanon ko pa kutob hit ak akos ngan sige la it ak pangadi nga kaluuyan ak hit Ginoo.”

Objective: Poor eye contact, glancing about

Activity and exercise

Subjective: Patient verbalized, “Ako man gud it nagi-os ha balay hit ngatanan nga trabahuun kay it ak anak ngan it iya asawa natrabaho man. Ako it naglilimpyo, nagluluto, may tindahan pa ako, tapos ako gihap it nag-aalaga hit ak mga apo. Maupay na ini nga exercise para ha akon.”

Nutrition and metabolism

Subjective: Patient verbalized, “Han una mahilig gud ak hin bisan ano nga magtam-is. Nakakaimod ak hin 500ml nga Coke adlaw-adlaw. Diri kumpleto tak adlaw kun diri ak nakakaon hin matam-is. Nakakatulo ak pagkaon ha usa ka adlaw: kan-on nga may sura ngan utan. Mahilig ak hin karne pero nakaon gihap ak hin isda. Nainom ak hin 8 ngadto 10 ka baso hin tubig ha usa ka adlaw. Kasabti nga mayda ko na diabetes, ginyaknan man ak han ak doktor nga kinahanglan magbag-o tak pagkaon, gintalinguha ko nga bagohon. Yana diri na ak nakaon hin duro nga magtam-is ngan duro nga magtambok nga pagkaon. Kun nakaon man ako, talagudti la. Kinhanglan ko disiplinahon tak kalugaringon. Napansin ko gihap nga baga la ako hin pirmi nauuhaw amu tagdaragmu na tak pag-inom hin tubig, mga 15-20 nga baso hin tubig. ”

Elimination

Subjective: Patient verbalized, “Waray problema tak pagbabawas, nakakausa ha usa ka adlaw. Tak pag-ihi lugod kay tagdaragmu, nakakaunom ha usa ka adlaw, bagan duro ka yellow it kolor tak ihi.”

Sleep and rest

Subjective: Patient verbalized, “Waray problema tak pagkaturog, unom ngadto pito ka-oras.”

Cognition and perception

Subjective: Patient verbalized, “Nakagraduate ak hin elementary pero waray na ak magapadayon pagskwela kay waray man kami kwarta. Maaram ak bumasa ngan sumurat. Nakakaintindi gihap ak hin tagalog ngan guti ng English. Yana, baga hin malabo naman it ak pagkita, pero maupay pa it ak pagbati. Waray pa liwat ak magpacheck-up ha doktor, diri ak maaram kun kinhanglan ko hin antiyuhos.”

Objective: Patient is responsive, has intact remote and recent memory, coherent, oriented to time, place, person.

Self-perception and self-concept

Subjective: Patient verbalized, “Maupay gad tak pagkita hit ak kalugaringon. Bisan diri kami riko, bisan waray ak magtapos hin college, ok la gihap kami, basta buhi la. Masisiring ko nga maupay an ak pagpatubo han ak mga anak. Ini nga akon kasakit, tungod na gihap ini han ak kawaray

Kimmelstiel-Wilson Syndrome Group O

disiplina han burubata pa ako, per okay adi naman ini, maguupay nala ak kutob hit ak kaya. Magaampo la ako ha Ginoo nga kaluuyan ako.”

Roles and relationships

Subjective: Patient verbalized, “Waray ako problema tak pakikitungo ha iba nga tawo. Waray ko man kaaway. Maupay tak relasyon hit ak mga anak, hit ira mga asawa, ngan hit akon mga apo ngan kasangkayan.”

Coping and stress management

Subjective: Patient verbalized, “Nakakalipay ha akon it ak mga apo. Kun ginkakapoy ako, mabatian ko la nga natawa hira, baga hin nawawara tak kapoy. Nalilibang liwat ako hit ak turutindahanay. Kun may oras it ak anak, nakadto kami ha beach kun sabado di ngani domingo para marelax.”

Sexuality and reproduction

Subjective: Patient verbalized, “Namatay naman an ak asawa han 2006 kay na heart attack. Buutan adto nga ak asawa, bisan nagkukuri kami nananalinguha gud la gihap hiya nga mayda kami kaunon ngan mayda isustento han am upat nga anak. Nakakamingaw gad usahay labis na kun nahinumdum ak han am mga mag-upay nga ginbubuhat hadto.”

Values and beliefs

Subjective: Patient verbalized, “Nasimba ako kada Domingo, nangangadi liwat ako adlaw-adlaw. Makusog man tak pagtuo hit Ginoo. Yana nga may sakit ako, duro tak pangadi nga buligan ako niya para makaya ko ini nga akon ginaagian yana. Nagdadahum pa gad ako nga matambal ako para humilaba pa tak kinabuhi ngan makabulig pa ha ak mga anak ngan mga apo.”

PHYSICAL EXAMINATION

General survey:

Patient Love is a 60 - year old female, anxious and weak but articulates clearly, has poor eye contact and was not in distress. Patient can ambulate but chooses not to because of her perceived weakness. She is fairly groomed and prefers lying in bed with head elevated to 45 degrees. Patient was examined conscious, coherent, oriented as to time, date, person and place. Weight: 140 lbs (64kg), Height: 5’4” (162.6m) BMI: 24

Vital signs:

Blood pressure:Blood pressure: 160/90 mmHg160/90 mmHg (left arm, lying) (left arm, lying) Temp:Temp: 37.5°C,37.5°C,

Pulse:Pulse: 66 bpm66 bpm Respiration: 20 cpm

Skin:

Inspection

Medium brown skin complexion. Pale No active lesions.

Palpation

With dry skin Senile skin turgor

Nails:

Inspection

Dirty untrimmed nails No clubbing of fingers.

Palpation

Smooth with convex curvature Pale With a slight delay in capillary refill >3 seconds.

Hair:

Inspection

Black and white, evenly distributed hair. No lice, no nits.

Head:

Inspection

Normocephalic No lesions, masses, injury, scars, lumps, scaling Symmetric facial features with no weakness, no involuntary movements observed.

Eyes:

Inspection

Symmetrical With symmetrically aligned eyebrows.

Kimmelstiel-Wilson Syndrome Group O

Eyelashes are equally distributed. Visual fields full by confrontation. EOMs intact, no nystagmus. No ptosis, lid lag, discharges, or crusting. Corneal light reflex symmetric, no strabismus. Pale palpebral conjunctivae. No periorbital edema. Sclera white, no active lesions or redness; no hemorrhage or exudates. Pupils are round and reactive to light and accommodation or PERRLA.

Ears:

Inspection

Symmetrical, equal sized auricles. Color is the same with facial skin. Auricle aligned to outer canthus of the eye. Tympanic membrane pearly gray, landmarks intact, no perforation. No hemorrhage, crusting or swelling. Cerumen noted on ear canals. Pinna has no mass, swelling, scaling, discharge.

Palpation

No lumps nor tenderness. Mobile and firm, pinna recoil after it is folded.

Nose:

Inspection

Pale nasal mucosa. Uniform with facial color. Nares patent. Nasal septum intact and at midline. Nose bridge not deviated. No perforation noted. No nasal discharges, no hemorrhage noted.

Palpation

No sinus tenderness. No lesions, no lumps or masses palpated.

Mouth:

Inspection

Pale, dry lips. Pale mucosa and gingivae.

With dental caries. With halitosis. Tongue protudes midline, no tremors. Pharynx without exudates. Uvula rises midline on phonation. Tonsils 1+. Gag reflex present. Dysphagia not noted. Voice hoarseness not noted.

Neck:

Inspection

No neck vein engorgement Muscles supple equal in size with full ROM; head centered. No masses, tenderness, lymphadenopathy.

Palpation

Trachea at midline. Thyroid nonpalpable, non-tender. No nodules noted.

Spine and back:

Inspection

Normal spinal profile. Vertebral column no tenderness, no deformity or curvature. No lateral spine deviation, no scoliosis, kyphosis, lordosis noted.

Palpation

No tenderness over spines. No CVA tenderness.

Percussion (Back)

Positive Kidney Punch

Thorax and Lungs:

Inspection

Symmetric chest expansion.

Palpation Symmetrical chest expansion.

Percussion

Tactile fremitus equal bilaterally.

Kimmelstiel-Wilson Syndrome Group O

Resonant in all lung fields. Diaphragmatic excursion bilaterally equal.

Auscultation

Bronchovesicular breath sounds in all regions, no crackles, no wheeze, no rhonchi.

Breast:

Inspection

Generally unequal in size. Uniform skin color and intact. Rounded, everted, equal in size and areola is dark brown in color, and points to the

same direction without discharges. Generally symmetric; no retractions or dimpling, no rashes, active lesions or

discharge. No active lesions noted.

Palpation

Soft and smooth. Contour and consistency firm and homogenous. No tenderness, masses or nodules noted.

Heart:

Auscultation

PMI in 5th left ICS at the midclavicular line; no thrills. No extra murmurs heard.

Abdomen:

Inspection

Skin with no scars or any active lesions. No visible veins.

Auscultation

Normoactive bowel sounds – 16 per minute. No bruits heard.

Percussion

Tympanic in all quadrants. Palpation

Soft, no organomegaly, no masses

Extremities:

Inspection

Tan in color, no active lesions noted. With Grade 2 pitting bipedal edema. Sole and nails are pale.

Palpation

Have minimal ROM Unable to maintain flexion against resistance. (Muscle strength: 3/5) All peripheral pulses present, 2+ and = bilaterally. No calf tenderness noted.

Neurologic:

Alert and oriented to person, place, time. Appearance, behavior, speech is appropriate. Remote and recent memories intact. Cranial nerves II through XII intact. Sensory: pinprick, light touch, vibration intact. Stereognosis, able to identify key.

REVIEW OF SYSTEMS

GENERAL SURVEY: Patient is pale and weak.

Integumentary:

Pale. Dry skin with Senile skin turgor. With edema No history of skin disease. No itchiness, no active skin lesions.

HEENT:

Head:

No unusually frequent or severe headaches, dizziness, syncope, or vertigo. Eyes:

Slightly blurred vision. 20/50 OD, 20/70 OS No eyeglasses. No eye pain or exudates. No redness.Ears:

Kimmelstiel-Wilson Syndrome Group O

No hearing difficulty. No earaches, infections now or as child, no discharge, tinnitus, or vertigo. Nose:

No discharges; had at least one cold per year. No sinus pain, nasal obstruction, epistaxis, or allergies to food and drugs.Mouth and throat:

With pale , dry lips. Pale mucosa and gingivae. With dental caries. With halitosis. No sores, no lesions. No gum bleeding. No dysphagia, hoarseness, nor sore throat.

Neck:

No pain, lumps or swollen glands. No stiffness.

Respiratory:

Eupneic.

Cardiovascular:

With palpitations. Hypertensive. With easy fatigability.

Gastrointestinal:

No vomiting. No diarrhea. Moves bowel everyday to a formed brown-colored stool. No pain nor rectal bleeding. No heartburn, no indigestion, no abdominal pain.

Genitourinary:

Voids yellow-colored urine approximately 1 glass (240ml) per void (6-10 times per day)

No pain during urination. No hematuria.

Peripheral Vascular:

With Grade 2 pitting bipedal edema.

No varicosities. No leg cramps.

Musculoskeletal:

With muscle weakness (Muscle strength: 3/5) With limitation of movement No joint pain, stiffness, muscle pain. No deformities.

Hematologic:

No easy bruising or bleeding.

Endocrine:

With excessive thirst. No heat/cold intolerance. No excessive sweat.

Psychiatric:

No flight of ideas, no shifting of mood

Neurologic:

No problems with coordination, difficulty speaking. No memory problems.

LABORATORY STUDIESClinical Chemistry:January 8, 2010

Normal Result Significance Serum creatinine 53-106 umol/L 111.17 ↑: decreased glumerular

filtration rate indicating deteriorating renal function

BUN 1.7 – 8.3 mmol/L 16.3 ↑: DM Nephropathy

Hgt 80 – 120 mg/dl 200 mg/dl ↑: Diabetes

Kimmelstiel-Wilson Syndrome Group O

Uric Acid 140 – 360 μmol/L 447.1 ↑: decreased glumerular filtration/ decreased tubular excretion of urate

Sodium 135 – 145 mEq/L 126.9 ↓: decreased Na reabsorption in tubule/ decreased glomerular filtration

Potassium 3.5 – 5 mEq/L 5.7 ↑: hyperkalemia; loss of excretory renal function

Urinalysis:January 7, 2010

Normal Result Significance

Glucose Negative Trace High blood glucose level

Albumin Negative ++ Albuminuria, may indicate damage to kidneys

Color Yellow Yellow normal

Transparency Clear Turbid Presence of crystals, RBCs, epithelial cells.

pH 5.0-9.0 5.0 normal

Specific gravity 1.010-1.035 1.010 normal

WBC 0-5/hpf 3/hpf normal

Epithelial Cells Few Moderate ↑: indicates tubular damage/ injury to the kidneys.

RBC 0 – 1/hpf 3/hpf ↑: Proteinuria

Bacteria Few Few normal

Hematology:January 7, 2010

Normal Result SignificanceHemoglobin 12 – 16 g/dL 9.7 ↓: anemia from failure to

produce erythropoietinHematocrit 37% - 47% 28 ↓: Low Hct leads to anemiaWBC 5 – 10 x 109 /L 9.1 x 109 /L NormalErythrocytes 4.6-6.2 x 1012/L 5.43 x 1012/L NormalLeukocytes 4.5-10 x 109/L 6.2 x 109/L NormalPlatelet 150-450 x 109/L 140 x 109/L Slightly decreased; partly due

to uremiaLymphocytes 0.15-0.35 0.10 ↓: anemia

24-Hour Urine Collection:January 8, 2010

Normal Result Significance

24-hour urine total volume

800 – 1800 mL 2800 ↑: polyuria

Urine creatinine 500 – 2000 mg/dl 182 ↓: creatinine is reabsorbed in kidney damage

Creatinine clearance

87 – 107 mL/min 48 ↓: inability to excrete creatinine in kidney damage

24-hour urine protein

150 mg/24hours 300 ↑:increased protein excretion in impaired kidney function

DIAGNOSTIC STUDIES

X-Ray:January 7, 2010

X-Ray Report:

Impression:No significant chest findings.

Ultrasound:January 9, 2010

Impression:

Glomerulonephritis

I. ANATOMY AND PHYSIOLOGY

Anatomy and Physiology of the Endocrine Pancreas

the pancreas weighs approximately 80 grams (about 3 ounces) and is shaped like a pear.

located in the upper abdomen, with the head lying immediately adjacent to the duodenum and the body and tail extending across the midline nearly to the spleen.

Only 1 to 2 percent of pancreatic tissue has endocrine function.

Kimmelstiel-Wilson Syndrome Group O

consists of the islets of Langerhans (approximately one million islets that weigh about one gram in total)

The principal function of the endocrine pancreas is the secretion of insulin and other polypeptide hormones necessary for the cellular storage or mobilization of glucose, amino acids, and triglycerides.

Pancreatic islets house three major cell types, each of which produces a different endocrine product:

Alpha cells (A cells) secrete the hormone glucagon. Beta cells (B cells) produce insulin and are the most abundant of the islet cells. Delta cells (D cells) secrete the hormone somatostatin, which is also produced by a

number of other endocrine cells in the body.

A. Glucagon

major role in maintaining normal concentrations of glucose in blood

has the effect of increasing blood glucose levels.

linear peptide of 29 amino acids. Glucagon is synthesized as proglucagon and proteolytically processed to yield glucagon within alpha cells of the pancreatic islets. Proglucagon is also expressed within the intestinal tract, where it is processed not into glucagon, but to a family of glucagon-like peptides (enteroglucagon).

Physiologic Effects of Glucagon

Glucagon exerts control over two pivotal metabolic pathways within the liver, leading that organ to dispense glucose to the rest of the body:

Glucagon stimulates breakdown of glycogen stored in the liver. When blood glucose levels are high, large amounts of glucose are taken up by the liver. Under the influence of insulin, much of this glucose is stored in the form of glycogen. Later, when blood glucose levels begin to fall, glucagon is secreted and acts on hepatocytes to activate the enzymes that depolymerize glycogen and release glucose.

Glucagon activates hepatic gluconeogenesis. Gluconeogenesis is the pathway by which non-hexose substrates such as amino acids are converted to glucose. As such, it provides another source of glucose for blood. This is especially important in animals like cats and sheep that don't absorb much if any glucose from the intestine - in these species, activation of gluconeogenic enzymes is the chief mechanism by which glucagon does its job.

Glucagon also appears to have a minor effect of enhancing lipolysis of triglyceride in adipose tissue, which could be viewed as an addition means of conserving blood glucose by providing fatty acid fuel to most cells.

Control of Glucagon Secretion

Two other conditions are known to trigger glucagon secretion:

Elevated blood levels of amino acids, as would be seen after consumption of a protein-rich meal: In this situation, glucagon would foster conversion of excess amino acids to glucose by enhancing gluconeogenesis. Since high blood levels of amino acids also stimulate insulin release, this would be a situation in which both insulin and glucagon are active.

Exercise: In this case, it is not clear whether the actual stimulus is exercise per se, or the accompanying exercise-induced depletion of glucose.

B. Insulin

Structure of Insulin

Insulin is a rather small protein, with a molecular weight of about 6000 Daltons. It is composed of two chains held together by disulfide bonds.

Biosynthesis of Insulin

Insulin is synthesized in significant quantities only in beta cells in the pancreas. The insulin mRNA is translated as a single chain precursor called preproinsulin, and removal of its signal peptide during insertion into the endoplasmic reticulum generates proinsulin.

Proinsulin consists of three domains: an amino-terminal B chain, a carboxy-terminal A chain and a connecting peptide in the middle known as the C peptide. Within the endoplasmic reticulum, proinsulin is exposed to several specific endopeptidases which excise the C peptide, thereby generating the mature form of insulin. Insulin and free C peptide are packaged in the Golgi into secretory granules which accumulate in the cytoplasm.

When the beta cell is appropriately stimulated, insulin is secreted from the cell by exocytosis and diffuses into islet capillary blood. C peptide is also secreted into blood, but has no known biological activity.

Control of Insulin Secretion

Glucose is transported into the beta cell by facilitated diffusion through a glucose transporter; elevated concentrations of glucose in extracellular fluid lead to elevated concentrations of glucose within the beta cell.

Elevated concentrations of glucose within the beta cell ultimately leads to membrane depolarization and an influx of extracellular calcium. The resulting increase in intracellular calcium is thought to be one of the primary triggers for exocytosis of insulin-containing secretory granules. The mechanisms by which elevated glucose levels within the beta cell cause depolarization is not clearly established, but seems to result from metabolism of glucose and other fuel molecules within the cell, perhaps sensed as an alteration of ATP:ADP ratio and transduced into alterations in membrane conductance.

Kimmelstiel-Wilson Syndrome Group O

Increased levels of glucose within beta cells also appears to activate calcium-independent pathways that participate in insulin secretion.

C. Somatostatin

first discovered in hypothalamic extracts and identified as a hormone that inhibited secretion of growth hormone.

Structure and Synthesis

Two forms of somatostatin are synthesized:

SS-14 -more potent in inhibiting glucagon release.

predominant form produced in the nervous system

the sole form secreted from pancreas

SS-28 - roughly ten-fold more potent in inhibition of growth hormone secretion

intestine secretes mostly SS-28

Both forms of somatostatin are generated by proteolytic cleavage of prosomatostatin, which itself is derived from preprosomatostatin. Two cysteine residules in SS-14 allow the peptide to form an internal disulfide bond.

Physiologic Effects

"somatostatin inhibits the secretion of many other hormones".

Effects on the Pituitary Gland

Somatostatin was named for its effect of inhibiting secretion of growth hormone from the pituitary gland.

Effects on the Pancreas

Somatostatin appears to act primarily in a paracrine manner to inhibit the secretion of both insulin and glucagon. It also has the effect in suppressing pancreatic exocrine secretions, by inhibiting cholecystokinin-stimulated enzyme secretion and secretin-stimulated bicarbonate secretion.

Effects on the Gastrointestinal Tract

It has been shown to inhibit secretion of many of the other GI hormones, including gastrin, cholecystokinin, secretin and vasoactive intestinal peptide. Somatostatin suppresses secretion of gastric acid and pepsin, lowers the rate of gastric emptying, and reduces smooth muscle contractions and blood flow within the intestine.

Effects on the Nervous System

Somatostatin is often referred to as having neuromodulatory activity within the central nervous sytem, and appears to have a variety of complex effects on neural transmission.

Renal System

I. The Kidneys

1. FunctionsA. Excretion of metabolic waste productsB. Regulation of the volume and composition of

extracellular fluids2. Produces Urine

A. Formed from bloodB. Varies in composition

II. Anatomy of the Renal System

1. KidneyA. Pair of Organs

a. Bean-Shaped--Most Speciesb. Heart-Shaped--Horsesc. Lobulated--Cattle

B. Retroperitoneala. Located outside of abdominal cavity suspended by peritoneum

C. Blood Supplya. Renal Artery

i. Brings blood to Kidneyii. Arises directly from aorta

b. Renal Veini. Brings blood away from Kidneyii. Empties into the Caudal Vena Cava

D. Divisionsa. Cortex

i. Outer Portionii. Contains upper parts of nephrons

--Glomeruli and Tubulesb. Medulla

i. Inner Portionii. Contains lower parts of nephrons--Loops of Henle and Collecting Ducts

c. Renal Pelvisi. Drains kidney and connects ureters

Kimmelstiel-Wilson Syndrome Group O

d. Renal Hilusi. Indented area on concave edgeii. Ureter, Blood Vessels, Nerves and Lymphatics enter or leave

2. UreterA. Smooth muscle lined tubeB. Conveys urine from renal pelvis to bladderC. Enter bladder through ureterovesicular junction

a. Acts as a valveb. Prevents backflow

3. Urinary BladderA. Hollow muscular organ (Smooth Muscle)B. Stores Urine

a. StretchesC. Lined with Transitional Epithelium

a. Flatten when filling occurs4. Urethra

A. Caudal continuation of the neck of the bladderB. Conveys urine from bladder to exteriorC. Urine release into urethra controlled by external sphincter

a. Skeletal muscle

III. Microstructure of the Kidney: The Nephron

A. The functional unit of the kidneya. Numbers range from 250,000 (cat) to 4 million (cow) per kidneyb. Components

i. Glomerulusii. Bowman's Capsuleiii. Proximal Tubulesiv. Loop of Henlev. Distal Tubules

B. Glomerulusa. Tufts of Capillariesb. Afferent arterioles

i. Conduct blood to glomerulusc. Efferent arterioles

i. Conduct blood away from glomerulusii. Divide into peritubular capillaries --Surround nephron tubules

--Vasa Recta: Peritubular capillaries surrounding loops of Henle

d. Function:Filtration of Blood

C. Bowman's Capsulea. Collect Glomerular Filtrateb. Conduct filtrate into proximal tubules

D. Proximal Tubulesa. Conduct filtrate into Loop of Henle

b. Very convoluted (Surrounded by Peritubular Capillaries)c. Reabsorb, Secrete, & Excrete

--Simple cuboidal or columnar epitheliumE. Loop of Henle

a. Play important role in concentration of urineb. Divisions

i. Descending limb--thin segment--simple squamous epithelium

ii. Ascending limb--thick segments--simple cuboidal epithelium--returns to sight near glomerulus

of originc. Surrounded by Vasa Recta

F. Distal tubulesa. Convey urine from Loops of Henleb. Also convoluted (Surrounded by peritubular

capillaries)c. Reabsorb, Secrete, and Excrete

--Simpled. Feed Collecting tubules and ducts

G. Juxtaglomerular Apparatusa. Junction between distal tube and glomerulusb. Contain unique cell types

i. Macula Densa in tubulesii. Juxtaglomerular cells in arterioles

c. Function:i. Regulate renal blood flowii. Regulate glomerular filtrationiii. Secrete Renin--Involved in production of angiotensin II

H. Two Types of Nephronsa. Cortical

i. Short loops of Henleii. Only go into Outer medulla

b. Juxtamedullaryi. Long loops of Henleii. Can go clear to renal pelvis

PATHOPHYSIOLOGY

Kimmelstiel-Wilson Syndrome Group O

PROGNOSIS

Our patient has a poor prognosis. This is mainly caused by the complications especially macrovascular diseases. In diabetic patients with proteinuria, a decline in the glomerular filtration rate, and hypertension, which collectively constitute the major clinical manifestations of Kimmelstiel-Wilson Syndrome, the mortality risk is 11-fold higher for diabetic men and 18-fold higher for diabetic women when compared with diabetics without nephropathy (our patient is a 60-year old female).

In type 1 diabetic patients with end-stage renal disease the patient survival rates could dramatically be improved mainly due to continuous hemodialysis and successful kidney transplantations. The prognosis of type 1 diabetic patients treated with hemodialysis is furthermore significantly poorer than that of kidney transplanted patients. During the last decade simultaneous pancreas and kidney transplantation has become the method of choice for these patients.

In type 2 diabetic patients (our patient) with microalbuminuria and overt proteinuria mortality is significantly increased, mainly due to cardiovascular disease. Several arteriosclerosis-related risk factors are more frequently seen in albuminuric patients, especially lipid abnormalities, and hemostatic disturbances. In type 2 diabetic patients with end-stage renal disease the patient survival rate is furthermore extremely low. The prognosis could be improved an inch by hemodialysis or kidney transplantation, however, during the last decade only 8% of type 2 diabetic patients who received either hemodialysis or kidney transplant survived a maximum of 3 years due to ESRD.

RECOMMENDATION

The principles of prevention and treatment of Kimmelstiel-Wilson Syndrome are the

same. However, the role of each factor could be different in each stage of disease. It is

important to define the stage that is the target of intervention (microalbuminuria, proteinuria or

GFR) and the outcome of interest. Two recent meta-analyses have demonstrated different

results when evaluating different outcomes, such as proteinuria, GFR decline or progression to

ESRD.

Probably the best treatment is a multiple risk factor interventional approach, but due to

a practical point of view each aspect will be addressed individually. The goal to be pursued is

retarding the development or progression of DN and to decrease the patient’s cardiovascular

risk and mortality.

1. Keep blood glucose levels as close to normal as possible.

The American Diabetes Association recommends that you keep your blood sugar levels at:

90 mg/dL to 130 mg/dL before meals 110 mg/dL to 150 mg/dL at bedtime.

ModifiableHypertension Diet

Non-modifiable-Heredity (66%)Gender (55% female)Race (Asian 15%)

Nephropathy

Insulin resistanceDiabetes Mellitus Type IINeuropathy

Hyperglycemia

RetinopathyGlomerulosc

lerosis

S/S:Grade 2 Pitting Bipedal EdemaPalenessPositive kidney punchPolyuria – 480cc/voidExcessive thirst

continuation (labs)…..24-HR Urine Collection:Volume – 2800mlUcr – 182 mg/dlCreatinine Clearance – 48 ml/min24-hr urine protein – 300mg/day

Renal Injury

Laboratory and Dx TestsHematology:Hgb – 9.7 g/dlHct – 28%Platelet – 140 x 109 /LLymphocyte – 0.10Clinical Chemistry:Serum Creatinine – 111.17umol/LBUN – 16.3 mmol/LHGT – 200mg/dlUric Acid – 447.1 umol/LNa – 126.9 mEq/LK – 5.7 mEq/LUrinalysis:Glucose – TraceAlbumin - ++Transparency – TurbidEpithelial Cells – moderateRBC – 3/hpfUTZ - Glomerulonephritis

Kimmelstiel-Wilson Syndrome Group O

Less than 180 mg/dL 1 to 2 hours after meals. Three keys to success in monitoring your blood sugar anywhere are:

Keeping your meter and supplies with you at all times so that you always have them when you need them.

Making it a habit to check your blood sugar level by building it into your routine. Checking your blood sugar meter's accuracy when you visit your doctor by comparing

your results with your doctor's results.

2. Maintain blood pressure at less than 130/80 mm Hg with medicine, diet, and exercise. 3. Follow the nutrition guidelines

Eating fewer processed foods, such as snack items, luncheon meats, and canned soups, will reduce the amount of sodium in your diet.

A diet high in calcium, potassium, and magnesium may lower your blood pressure. A diet high in sodium may cause high blood pressure. To increase the potassium in your diet, fruits and vegetables are excellent sources of

this nutrient. Dairy products are high in calcium and magnesium. DASH recommends that you eat 8 to 10 servings of fruits and vegetables and 3 servings of low-fat dairy products each day.

Eating a diet low in both saturated fat and total fat will also help lower your blood pressure. Only 30% of your total calories should be from fat, with only 7% to 10% of your fat calories from saturated fat. Saturated fat is found in meats, cheeses, butter, poultry, snack foods, and other processed foods.

In general, vegetarian diets reduce blood pressure. The DASH diet could easily be a vegetarian diet if legumes were substituted for meat. Vegetarian diets tend to be higher in potassium, magnesium, and calcium, as is the DASH diet. Vegetarian diets also are higher in fiber and unsaturated fat than other diets.

4. Do not smoke or use other tobacco products.

5. Avoid dehydration by promptly treating other conditions—such as diarrhea, vomiting, or fever—that can cause it.

6. Treat other conditions that may block the normal flow of urine out of the kidneys , such as kidney stones, an enlarged prostate, or bladder problems.

7. Avoid the use of unprescribed medicines that may be harmful to our kidneys, especially nonsteroidal anti-inflammatory drugs (NSAIDs).

Kimmelstiel-Wilson Syndrome is a chronic complication of DM with a growing

incidence. Therefore it is essential to have a better understanding of it, especially in relation to

prevention and aggressive management to avoid progression to ESRD. Besides, its direct

association with cardiovascular complications makes it imperative to perform intensive, early

management of the risk factors. The study of DN has evolved a lot as regards its

pathophysiology, stages of renal involvement and, especially, the therapeutic instruments

available. Early detection of DN, and the multi-factorial approach targeting the main risk factors

may delay progression of kidney disease in DM.