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Prestige Medical News
Tel: (914) 682-2080
EDITORIAL BOARD
Editor-in Chief
Valiere Alcena, MD, FACP, FAAC
______
Juanita Alcena, BA, MA
Betty R. Arellano, MSW, CSW, DSW, BC, MPH
Alison Berry, ESQ
Delores Brown-Hall, RN, MS , PHD
Glenn A. Davis, MD
Delores Gayle-Thompson, MD, MPH
DianaLake, MD, FACP
Jamesatta A. Newland, MS, RN, CS, FNP, PHD
Fredrick Strauss, MA
A Quarterly Medical Journal Published by:
Prestige Medical News
37 Davis Avenue
White Plains, NY10605
RICKETS TYPE – I IN A SUBURBANHOSPITAL: AN UNUSUAL PRESENTATION
Valiere Alcena M.D. F.A.C.P., F.A.A.C., Rhonda Rubin M.D., Meyer Halberstam M.D.
LE is a 78 year old female who was brought into a Suburban Emergency Room, in Westchester, New York with complaints of failure to thrive. The family of Ms. E had noticed two days of increased lethargy and worsening gait. Earlier on the day of admission, they noticed a facial droop. Ms. E.’s diet had changed to one consisting of mostly fruits and vegetables with a limited amount of ensure since her husband’s death several months before.
Past medical history consisted of mild dementia and hypertension. There was no history of thyroid disease, diabetes mellitus or pancreatitis.
Her surgical history was unremarkable.
Medications at home: Amiloride/HCTZ 5/50-mg daily, Aricept 10-mg daily and vitamin B complex and folate.
Internist: A complete disruption of cranial nerve VII leads to paralyzes of all muscles of facial expression. The corner of the mouth droops, creases and skin folds disappear and the eyelids will not close. What this patient had was a partial VII nerve palsy. The most common cause of the paralysis is Bell’s palsy. But as she had other generalized complaints e.g. lethargy and gait disturbance, other causes of partial face palsy need to be considered. The differential diagnosis includes:
Tumors such as cholesteatomas, acoustic neuromas
Infections, most commonly Herpes Zoster (Ramsay Hunt Syndrome),
Leprosy
Infarction in the pons
Nutritional. The CNS depends upon carbohydrates and is therefore extremely sensitive to nutritional deficiencies especially of the Vitamin B vitamins. Wernicke’s (thiamine deficiency) leads to ataxia, apathetic dementia but notable for ocular motor deficiency.
Subacute combined degeneration (vitamin B12) deficiency begins with general weakness and paresthesias. The legs are the main site of motor defects.
In the ER, the patient was in no acute distress though appeared poorly nourished. She was afebrile with a blood pressure of 120/70 and a pulse of 97. Her pulse oxygenation showed 95% saturation with 2L of O2 on nasal cannula. Ms. E. physical examination was unremarkable. Her lungs were clear; the heart had normal rate and sounds. The abdomen was benign, with no tenderness, masses or bruits appreciated. There was no pedal edema. Her neurological examination was notable only for the facial droop. She had no defects of the 6th nerve. Her mental status was awake, alert and at baseline.
The laboratories values were as follows:
ABG on 2LPM of O2: 7.37/42/119
16.8 WBC with left shift, HCT of 53 (HGB 17.2) and a platelet count of 168.
Glucose: 508, Sodium 150 (corrected: 156), Potassium 6.4, Chloride 113, Bicarb 27 with a BUN of 200 and a Creatinine of 2.9. The Calcium was 8.2, Albumin 4.1 and Alkaline Phosphatase of 574.
Urinalysis: 1.020, pH 5, trace protein, negative heme, benign sediment.
CAT scan of head: mild atrophy
Chest X-ray was unremarkable
EKG: Regular sinus rhythm with marked peaked T waves
Nephrologist: The patient had developed acute renal failure with moderate hyperkalemia but no metabolic acidosis. ARF develops from either decreased renal perfusion (prerenal azotemia), renal parenchyma disorders (Renal) or obstruction (postrenal).
Ms. LE has a picture of prerenal azotemia, with a BUN: Creatinine ratio of 69:1 and benign sediment. Decreases in renal perfusion is caused by: extracellular fluid loss (bleeding, burns, diuresis from hyperglycemia or diuretics, diarrhea or third spacing as seen in peritonitis, pancreatitis); decreased cardiac output (CHF), peripheral vasodilatation (sepsis), renal vasoconstriction(sepsis, liver disease, medications e.g. NSAID, ACE-inhibitors).
The most likely etiology of the acute renal failure is volume depletion from her new onset diabetes mellitus. She is hypernatremic and hyperchloremic with a hematocrit of 53%. Her kidneys are holding onto sodium and chloride in attempts to expand her intravascular volume. She is still able to acidify her urine and avoid metabolic acidosis which indicates no significant renal damage so far.
Sepsis as a cause of her hyperglycemia is a strong possibility with the elevated WBC, though this patient has no history of diabetes mellitus.
Of note, is the patient’s elevated alkaline phosphatase level. Alkaline phosphatase is an enzyme that correlates with mineralization by osteoblasts, hepatic infiltrates such as carcinoma or virus and cholestasis. She has no other clinical signs or symptoms of liver disease.
Hospital course: The patient was treated with IV hydration, kayexalate and insulin. Levaquin was administered pending cultures. Her hyperglycemia, acute renal insufficiency and hyperkalemia quickly resolved. The treatment unmasked a life-threatening hypocalcemia (Table 1).
Table 1:
Day / 3 / 4 / 5 / 6 / 7 / 8* / 9 / 10 / 11 / 12 / 13 / 14Alk Phos / 489 / 438 / 441 / 455 / 575 / 603 / 574 / 605 / 588 / 566 / 457
Calcium / 7.5 / 6.5 / 5.6 / 4.6 / 3.5 / 3.9 / 3.8 / 3.7 / 4.3 / 4.9 / 5.5 / 5.7
Albumin / 3.2 / 2.7 / 2.6 / 2.6 / 2.6 / 2.3 / 2.5 / 2.3 / 2.4 / 2.2 / 2.2 / 2.2
Magnesium / 0.7 / 1.1 / 1.1 / 0.9 / 1.2 / 1.2 / 1.4 / 1.6
Phosphorus / 3.6 / 2.6 / 1.8
PTH / 1001 / 837
*Vitamin D, 25-OH: < 5 (9 – 54 ng/mL)
*Vitamin D 1, 25 – D- OH: 19 (19 – 67 pg/mL)
Endocrinologist: Ms. LE is a 78 year old female with a history of mild dementia and hypertension, became malnourished after the death of her husband, who had been the primary care giver. The patient had classic signs of Vitamin D-Dependent Rickets Type I.
She had hypocalcemia, hypophosphatemia, secondary hyperparathyroidism and increased alkaline phosphatase. Serum evaluation revealed non-existent levels of Vitamin D, 25-OH and low levels of Vitamin D 1, 25 – D- OH. Vitamin D deficiency usually occurs as a result of decreased intake and absorption often exacerbated by decreased sun exposure (1,2). In the elderly it is particularly common due to an associated decline in cutaneous vitamin D production secondary to atrophic skin, a less varied diet which is usually lacking in vitamin D and changes in clothing.
Vitamin D deficiency is defined as the lowest threshold value for 25-OH that presents with secondary hyperparathyroidism (3). It is suggested that absolute levels of 25-OH vitamin D ranging from 12-25 nmole//liter presents with proximal myopathy (5) and levels of < 10-12 nmoles/liter have frank osteomalacia (6). Patients need at least 400 IU of Vitamin D intake a day.
Of particular interest was this patient’s presentation with hyperglycemia. There has been strong association between vitamin D levels and the development of diabetes mellitus. Studies have shown that vitamin D supplementation during childhood may reduce the risk of late development of Type I diabetes mellitus (7,8,9). While others have demonstrated an association between Type II diabetes mellitus and vitamin D deficiency. Both animal and human studies have shown that vitamin D deficiency inhibits insulin secretion (10, 11), Beta cell function and glucose tolerance. The pancreatic Beta cells have in fact exhibited vitamin D receptors (12). Interestingly, it is well known that there is improved glycemic control and lower Hgb A1-C during the summer months (13) when presumably patients are getting more outdoor exposure with concomitant increases in vitamin D levels. A secondary mechanism appears to be that PTH elevation causes deterioration in insulin sensitivity and decreased secretion.
The patient was unable to ingest sufficient calories and a PEG tube was placed. LE received 3 grams of Calcium gluconate IV daily, 1 gram of calcium carbonate po daily, Vitamin D 400 IU po daily and 2 grams of Magnesium sulfate IV daily in order to correct her electrolytes.She was able to be discharged to a skilled nursing facility.
Reference:
1. Glerup, H., Milokelsen, K., Poulsen, L., Hass, E., Overbeck,S., Thomsen, J., Charles, P. and Eriksen, E.F. (2000) Commonly recommended daily intake of Vitamin D is not sufficient if sunlight exposure is limited.
Journal of Internal Medicine, 247: 260-268.
2. Iqhal, S.J., Kuddam, I., Wassif, W., Nichol, G. and Walls, J. (1994) Continuing clinically severe vitamin D deficiency in Asians in the UK.
Postgraduate Medical Journal, 70: 708-714.
3. Holick, M.P. (1999) Vitamin D: photobiology, metabolism, mechanism of action and clinical application. In: Gavus, M.J. editor. Primer on Metabolic Bone Diseases and Mineral Metabolism. 4th edition
Lippincott, Williams and Wilkins, Philadelphia, 92 – 98.
4. Holick, M. P., Matsuoka, L.Y. and Wortsman, J. (1989) Age, Vitamin D and solar ultraviolet radiation.
Lancet, 4: 1104-1105.
5. Glerup, H. (1999) Investigations on the role of vitamin D in muscle function. A study of muscle function in Vitamin D deficient humans and the effect of treatment with Vitamin D. PhD thesis, Aarhus University, Denmark.
6. Hunchette, O.L. and Schwartz, G.G. (1992) Geographic patterns of prostate cancer mortality: Evidence for a protectice effect of ultraviolet radiation.
Cancer, 70: 2861-2869.
7. The Eurodiab Substudy 2 Study Group (1999) Vitamin D supplementation in early childhood and the risk for type I Insulin dependent diabetes mellitus.
Diabetologia, 42: 5154.
8. Hypponen, E., Laara, E., Reunanen, A., Jarvelin, M.R. andVirtanen, S.M. (2001) Intake of Vitamin D and incidence of type I diabetes: a birth cohort study.
Lancet, 358: 1500 – 1503.
9. Stener, L.C. and Joner, G. (2003) Use of cod liver oil during the first year of life is associated with a lower risk of childhood-onset type I diabetes: a large population based care control study.
American Journal of Clinical Nutrition, 78: 1128 – 1134.
10. Norman, A.W., Frankel, B.J., IIeldt, A.M. and Godsky, G.M. (1980) Vitamin D deficiency inhibits pancreatic secretion of insulin.
Science 209: 823 – 825.
11. Boucher, B.J. (1998) Inadequate Vitamin D status: does it contribute to the disorders comprising syndrome X?
British Journal of Nutrition, 79: 315-327.
12. Brown, A.J., Dusso, A. and Slatopolsky, E. (1999) Vitamin D.
American Journal of Physiology/ Renal Physiology, 277: F157- F175.
13. Ishii, H., Suzuli, H., Baba, T., Nakamura, K. and Watanabe, T. (2001) Seasonal effects of glycemic control in type 2 diabetic patients.
Diabetes Care, 24: 1503.
1. Vitamin D deficiency is a cause of hyperglycemia
2. Hyperparathyroidism inhibits Insulin Release
3. Pancreatic Beta cells have vitamin D receptors
4. Alkaline Phosphatase can be a sign of osteoblast function
5. Malnutrition an under appreciated problem
From the Department of Medicine
MontefioreHospitalMedicalCenter of
AlbertEinsteinCollege of Medicine
211 East 210th Street, Bronx,New York.
Dr Alcena is Clinical Professor of Medicine at
AlbertEinsteinCollege of Medicine, Bronx, New York.
Dr Rhonda Rubin is an Assistant Clinical Professor of Medicine at
AlbertEinsteinCollege of Medicine, Bronx, New York.
Dr Meyer Halberstam is an Assistant Professor of Medicine at
AlbertEinsteinCollege of Medicine, Bronx, New York.
Send Correspondence to:
Valiere Alcena, M.D. F.A.C.P., F.A.A.C
37 Davis Avenue
White Plains, NY10605
(914) 682-8020
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