Gram-negative rods

     MacConkey's agar

1) Lactose fermenter --- pink

-------fast lactose fermenter ---------

Klebsiella

E.coli

Enterobacter

-------slow lactose fermenter--------

Citrobacter

Serratia

2) Lactose non-fermenter-----white

A. ---------Oxidase negative--------

NO H2S production----------------------Shigella

H2S production--------------Salmonella

                          --------------Proteus

B. ---------Oxidase positive---------

                   P.aureginosa

Hypothalamic nuclei

Ventromedial ===> satiety, stimulated by leptin (dysphagia)

Lateral ====> hunger, inhibited by leptin (anorexia)

Anterior =====> heat dissipation (hyperthermia)

Posterior ======> heat conservation (hypothermia)

Arcuate ======> dopamin secretion, GHRH, gonadotropin

Paraventricular =====> ADH, CTRH, oxytocin, TRH

Supraoptic =====> secretion of ADH, oxytocin

Suprachiasmatic =====> circadian rythm, pineal gland function

PHaryngeal arch 1 --- Aortic arch 1---- CN V (Trigeminal n)-- Maxilary artery

PHaryngeal arch 2 --- Aortic arch 2---- CN VII (Facial n)-- Stapedial artery 

PHaryngeal arch 3 --- Aortic arch 3---- CN IX (Glossopharyngeal n)-- Common  carotid artery, Proximal internal carotid 

PHaryngeal arch 4 --- Aortic arch 4---- CN X (Sup Laryngeal branch of      Vagus)-- True aortic arch, subclavian arteries

PHaryngeal arch 6 --- Aortic arch 6---- CN X (Recurrent laryngeal branch of
                                                                 Vagus)-- Maxilary artery Pulmonary                     
                                                                  arteries, Ductus arteriosus

CN III - oculomotor nerve

Innervates:
 muscles:
- superior, middle, inferior rectus
-  inferior oblique
- levator palpebra superioris

Functions:
 - adduction
- depression
- elevation
- external rotation

Lesions:
- eye deviated downward & laterally
- diagonal diplopia
- dilation of pupil
- loss of accommodation
- ptosis

Dysthymic disorder

Depressed mood for most of the days for AT LEAST 2 years.

There is presence of AT LEAST 2 symptoms of the following:
1) poor appetite
2) overeating
3) insomnia
4) hypersomnia
5) fatigue
6) low self-esteem
7) poor concentration
8) difficulty making decision
9) feeling of hopelessness

17alpha-hydroxylase

---- LOW cortisol
---- LOW testosterone

---- HIGH mineralocorticoids
---- HIGH corticosterone (weak glucocorticoid)

---All patients phenotypically female
---Fluid and salt retention 
----hypertension

11beta-hydroxylase

--- LOW cortisol
--- LOW aldosterone

--- HIGH  testosteron
--- HIGH 11-deoxycorticosterone
--- HIGH 11-deoxycortison

--- ambigous genitalia in girls
--- fluid and salt retention
--- hypertension

21-hydroxylase deficiency

--- LOW cortisol
--- LOW aldosterone


--- HIGH  testosterone
--- HIGH 17-hydroxyprogesterone

---ambiguous genitalia in GILRS
--- salt wasting (vomiting, HYPOtension, LOW Na, HIGH K)

Infections in patients with chronic granulomatous disease

Patient with chronic granulomatous disease (mutation NADPH oxidase) are at increased risk of having infections caused by:
--- Staph aureus
--- Aspergillus
--- Nocardia
--- Serratia marcescens
--- Pseudomonas capacia

Celiac disease

increased sensitivity to gluten.

Ca ----------- LOW

Phosphorus  ------------ LOW

PTH ---------   HIGH

Type Ia Glycogen Storage Disease --- von Gierke disease

In this disease body cannot break down glycogen for energy.
G-6-P deficiency is the specific enzyme deficiency in von Gierke disease.
Von Gierke disease is an autosomal-recessive condition.
Deficiency of G-6-P blocks the final steps of glycogenolysis and gluconeogenesis. This results in severe hypoglycemia.

---Severe fasting hypoglycemia
---HIGH level of glycogen in liver
---higl level of lactate
---hepatomegaly
---seizures
--- kidny stones
---gout
---hypotonia in infants
---xythomas on the buttoks
---short stature if left untreated

Ornithine Transcarbamylase Deficiency

------ an X-linked genetic disorder of the urea cycle that leads to elevated levels of ammonia in the blood because of body's inability to eliminate ammonia.
------ orotic acid in blood in urine
------ decreased BUN

• Anorexia
• Irritability
• Heavy or rapid breathing
• Lethargy
• Vomiting
• Disorientation
• Somnolence
• Asterixis (rare)
• Combativeness
• Obtundation
• Coma
• Cerebral edema
• Death
  
  
  It may occur in any age. 
  
  
  Neurologic symptoms:
  
  
  • Poor coordination
  • Dysdiadochokinesia
  • Hypotonia or hypertonia
  • Ataxia
  • Tremor
  • Seizures and hypothermia
  • Lethargy that progresses to combativeness, obtundation, and coma
  • Decorticate or decerebrate posturing
    
    
    
    
    Treatment:
    ---Immediate temporary discontinuation of protein intake.
    ---Reduction of high level of blood ammonia can be achieved with hemodialysis.
    ---IV: sodium benzoate, sodium phenylacetate, arginine
    
    
    
    

Urea cycle